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PURPOSE: Phase 3 icatibant trials showed that most hereditary angioedema (HAE) (C1 inhibitor deficiency) acute attacks were treated successfully with one injection of icatibant, a selective bradykinin B2 receptor antagonist. We conducted a post hoc analysis of icatibant reinjection for HAE type I and II attacks in a real-world setting by using data from the Icatibant Outcome Survey, an ongoing observational study that monitors the safety and effectiveness of icatibant treatment.METHODS: Descriptive retrospective analyses of icatibant reinjection were performed on Icatibant Outcome Survey data (February 2008 to December 2012). New attacks were defined as the onset of new symptoms after full resolution of the previous attack. Potential associations between the patient and attack characteristics and reinjection were explored by using logistic regression analysis.RESULTS: Icatibant was administered for 652 attacks in 170 patients with HAE type I or II. Most attacks (89.1%) were treated with a single icatibant injection. For attacks that required two or three injections, the second injection was given a median of 11.0 hours after the first injection, with 90.4% of second injections administered ≥6 hours after the first injection. Time to resolution and attack duration were significantly longer for two or three injections versus one icatibant injection (p < 0.0001 and p < 0.05, respectively). Multivariate logistic regression analysis identified sex, attack severity, and laryngeal attacks as significantly correlated with reinjection (all p ≤ 0.05). These factors did not remain predictors for reinjection when two outlier patients with distinct patterns of icatibant use were excluded.CONCLUSIONS: In this real-world setting, most HAE attacks resolved with one icatibant injection. There was no distinct profile for patients or attacks that required reinjection when outliers with substantially different patterns of use were excluded. Because new attacks were not distinguished from the recurrence of symptoms, reinjection rates may be slightly higher than shown here. Clinical trial identifier: NCT01034969.

Bone mass is primarily genetically determined, but exogenous factors also play a major role. The prevention of osteoporosis can start from childhood, and optimal achievement of peak bone mass during childhood and adolescence is important in order to minimise future fracture risks. Chronic inflammatory diseases can have a detrimental effect on bone mass, by means of several mechanisms. Different diagnostic methods for detection and monitoring of osteoporosis are in use or under investigation. The role of calcium and vitamin D supplementation for the prevention and treatment of osteoporosis associated with paediatric rheumatic diseases remains to be established. New treatments such as bisphosphonates and calcitonin are now available, although their use in the paediatric age has been limited.

Resume Introduction. – Le syndrome de Sjogren est la plus frequente des maladies auto-immunes. Les manifestations neurologiques sont polymorphes et parfois revelatrices. L’epilepsie et l’atteinte du systeme nerveux autonome sont moins connues et probablement sous estimees. Exegese. – Nous rapportons le cas d’une patiente qui a presente un syndrome de Sjogren avec atteinte neurologique diffuse comportant des troubles moteurs, une dysautonomie et une epilepsie. Conclusion. – Le syndrome de Sjogren doit etre systematiquement recherche devant tout tableau neurologique complexe et notamment en presence d’une dysautonomie.

Subarachnoid hemorrhage is the most common cause of cerebral salt wasting syndrome. There are few reports of this condition in infectious meningitis. We describe a patient with hyponatremia and bacterial meningitis. Hyponatremia rapidly improved after administration of sodium chloride. The purpose of this report is to alert clinicians to the fact that hyponatremic patients with central nervous system disease do not necessarily have a syndrome of inappropriate secretion of antidiuretic hormone (SIADH), but may have cerebral salt wasting syndrome. By contrast with SIADH, the treatment requires saline administration.

INTRODUCTION Familial occurrence of progressive systemic sclerosis is unusual. The occurrence of conjugal scleroderma is exceptional. EXEGESIS We report here a case of systemic sclerosis in a wife and husband who both developed the onset of illness within a 10-year period. Solvent exposure was noted. CONCLUSION The etiology of systemic sclerosis remains unknown. Environmental factors may play role in its pathogenesis.

Resume Afin d'etudier la relation entre d'une part la presence de manifestations cliniques evocatrices d'allergie ou les resultats de tests cutanes vis-a-vis des pneumallergenes et, d'autre part, le niveau d'immunodeficience clinique et biologique au cours de l'infection par le VIH, une etude transversale a ete mise en place. Pour chaque patient, un entretien standardise sur les manifestations cliniques evocatrices d'allergie (MCEA) recentes, des tests cutanes a six pneumallergenes communs (pricks) et un dosage des IgE totales etaient realises. Parmi les 115 patients inclus, la moyenne des lymphocytes CD4+ (CD4) etait de 214,7/mm3 (etendue: 0-1 328/mm3). Des MCEA etaient retrouvees chez 8,8 % des sujets ayant un nombre de CD4 inferieur ou egal a 50, chez 30 % des sujets ayant des CD4 entre 51 et 200, chez 36 % des sujets ayant des CD4 entre 201 et 350 et chez 11,5 % des sujets ayant plus de 350 CD4 (p=0,03). Pour les patients ayant entre 51 et 350 CD4, le risque de presenter des MCEA etait 4,8 fois plus eleve que pour les autres patients (intervalle de confiance a 95 %=1,7–13,5) (p=0,003). Il n'y avait pas de difference de prevalence des tests cutanes positifs selon le niveau de CD4. Les MCEA sont plus frequentes quand le taux de CD4 est compris entre 51 et 350, evoquant un terrain allergique acquis au cours de l'infection par le VIH. Le mecanisme physiopathologique expliquant la moindre frequence des MCEA quand l'immunodepression est profonde (CD4 inferieurs ou egaux a 50) reste a preciser.

Resume Introduction Le cadmium est une substance cancerogene connue impliquee dans des neoplasies bronchiques et prostatiques. C'est aussi un produit nephrotoxique et sa responsabilite dans la survenue de cancers de rein a ete suspectee mais tres peu de cas etayant cette hypothese ont ete publies dans la litterature. Exegese Nous rapportons l'observation d'une femme de 59 ans soumise pendant 30 ans a une exposition professionnelle au cadmium. Elle a presente un carcinome epidermoide du rein et les dosages plasmatiques du cadmium effectues alors ont confirme l'existence d'une contamination chronique puis, 3 ans plus tard, un cancer bronchique. Conclusion La notion d'une exposition prolongee au cadmium, sa nephrotoxicite, la rarete des cancers epidermoides du rein et la survenue d'un autre cancer induit nous ont fait retenir la responsabilite de cette substance dans la neoplasie renale de notre malade. Cette hypothese a ete admise par la Commission de reconnaissance des maladies professionnelles.

Background: The safety and efficacy of sublingual-swallow immunotherapy (SLIT) in rhinitis caused by house-dust mite were evaluated in a double-blind, placebo-controlled study including 75 patients for 24 months. Methods: Patients received either placebo or SLIT with a standardized Dermatophagoides pteronyssinus (D.pt.) –D. farinae (D.f.) 50/50 extract. The mean cumulative dose was 90 000 IR, equivalent to 2.2 mg of Der p 1 and 1.7 mg of Der f I. Symptom and medication scores were assessed throughout the study. Exposure to house-dust mite, skin sensitivity, and serum specific IgE and IgG4 were assessed before starting treatment and after 12 and 24 months. Results: Seventy-two patients (36 active–36 placebo) were eligible for intent-to-treat analysis. Thirty-six patients dropped out of the study. The number of patients who dropped out due to lack of efficacy was eight out of 37 (21.6%) in the active treatment group compared to 15 out of 38 (39.5%) in the placebo group (chi-square=2.81, P=0.09). Total symptom and medication scores decreased significantly after 12 and 24 months (P

The hyper-IgD syndrome (HIDS) is an autosomal recessively inherited auto-inflammatory syndrome, caused by deficient enzyme activity of mevalonate kinase (MVK), an enzyme in the isoprenoid pathway. Patients present with a long history of recurrent fever attacks, accompanied by abdominal pain, skin lesions, lymphadenopathy and arthralgia. The attacks last about 3–7 days followed by a spontaneous remission, only to recur after a symptom-free period of 4–6 weeks. Factors known to precipitate an attack are infection, trauma, vaccination, and psychological stress. Laboratory examination at the time of attacks reveals a vigorous acute phase response with leucocytosis, raised sedimentation rate, high serum concentrations of C-reactive protein and increased mevalonic acid urinary levels. Almost all patients have an elevated polyclonal immunoglobulin D. Until now, treatment of HIDS patients is largely supportive and very difficult. Various anti-inflammatory drugs have failed to suppress the fever attacks [1].

Aspirine-sensitive asthma (ASA) is a disease defined only by clinical criteria. It is an intrinsic asthma related to a hypersensitivity to aspirin. The illness is linked to abnormalities in platelet and macrophage arachidonic acid metabolism. We assessed in vitro the platelet chemoluminescence (CL) and lymphocyte proliferative response of ASA patients. We observed that platelets from patients and control do not generate any CL in the presence of aspirin. Concerning the proliferative response of lymphocytes, the in vitro effect of aspirin depends upon the origin of the lymphocytes tested. Thus aspirin clearly enhances the proliferative response of lymphocytes from normal subjects but diminishes the thymidine uptake by lymphocytes from ASA patients. This discrepancy in the in vitro response of lymphocytes from normal subjects and patients might be useful for in vitro diagnosis of ASA.

A 49-year-old woman presented with isolated aphasia followed by dementia and ataxia with a duration of 4 years. Histopathologically there was panencephalic involvement, status spongiosus, and kuru-type plaques. Molecular analysis showed heterozygosity at codon 129 in the prion protein (PrP) gene, and type 2 protease-resistant PrP. The comparison between this case and those previously reported suggests that the panencephalopathic variant of Creutzfeldt-Jakob disease (CJD) is an aspecific end-stage condition displayed by most if not all CJD variants in individual patients with an unusually prolonged course.

The peritoneal equilibration test (PET) is routinely performed in adults treated with chronic peritoneal dialysis to assess the peritoneal transport rate and to optimize treatment prescription. Only a few and not well-standardized studies on the PET have been reported in children. Twenty-six PETs were performed In 16 children, mean age 10.9±4.9 years, mean body weight (BW) 26.8±11.9 kg, treated with nightly intermittent peritoneal dialysis (NIPD). The PET was performed according to Twardowski. Forty mL/kg BW of 2.27% glucose solution were Infused at a rate of 10 mL/kg BW every 2 minutes. In the simplified test, dialysate samples were taken at dwell times 0, 2 and 4 hours for glucose and creatinine. A blood sample was taken after a 2-hour dwell time for the same parameters. The standard PET (8 patients) consisted of dialysate samples at 0, 15, 30, 60, 120, and 240 minutes and blood samples at 0 and 240 minutes. Mean DIP ratio for corrected creatinine and DIDo ratio for glucose at 2 hours were 51.6±11.6 and 50.4±9, respectively; at 4 hours 69.6± 12 and 34.4±9.8, respectively. There was good correlation between DIP creatinine and DIDo glucose at 4 hours (p In conclusion, the PET, modified for use in children, gave reliable and reproducible results. Mean values of DIP creatinine for children were higher and DIDo glucose lower than those of adults as reported by Twardowskl. A consistent percentage of children had, nevertheless, a low-average or low transport rate. Standard PET is required in case of automated peritoneal dialysis prescription.

We report the case of a right atrial-ventricular chamber thrombus discovered four years after insertion of a Le Veen shunt for treatment of refractory ascites. A two-dimensional echocardiogram, performed after the discovery of an isolated systolic murmur, demonstrated a "tumorlike" mass seated in both the right atrium and the right ventricle. The mass was surgically removed and histologic examination confirmed that it was a thrombus developed at the tip of the catheter. We propose that two-dimensional echocardiography should be performed periodically for the survey of such intracardiac devices.